Sjögren’s syndrome

Definition

Sjögren’s syndrome (also called Sjögren-Larsson syndrome) was first described in 1933 by the Swedish ophthalmologist Henrik Sjögren. It is a dryness of the eyes and the oral cavity in connection with a rheumatic disease, chronic polyarthritis.

Introduction

Sjögren’s syndrome is an autoimmune disease first described in 1933, in which the body’s immune system is directed primarily against salivary and lacrimal glands. It belongs to the so-called collagenoses. Sjögren’s syndrome causes symptoms such as The disease is detected by means of a blood test in which autoantibodies, i.e. antibodies against the patient’s own body, are detected.

The treatment is still difficult today due to the unexplained causation of the disease. The complaints are treated: Eye drops against dry eyes, drink a lot against dry mouth, painkillers and for painful joint involvement. In addition, anti-inflammatory drugs can also be used.

The prognosis is generally good, but depends on the accompanying diseases.

  • Dry eye (leading symptom),
  • Dry mucous membranes in the mouth, nose and throat area and
  • Joint complaints with themselves. The cause of the occurrence of this disease is still unknown.

What is the classification of Sjögren’s syndrome?

If diseases such as lymphoma, AIDS, sarcoidosis or graft-versus-host disease are present, these classification criteria are not used. If the diseases just mentioned are not present and no other rheumatic diseases are known, it can be assumed with a probability of over 90% that a primary Sjögren’s syndrome is present as soon as 4 of the 6 criteria are fulfilled (in the case of point 6, only the presence of SS-A/Ro antibodies is obligatory). If a further (rheumatic) disease (such as rheumatoid arthritis (chronic polyarthritis), lupus erythematosus or scleroderma) is known, there is an over 90% probability that a secondary Sjögren’s syndrome exists if the first or second criterion and the sixth criterion and two of the criteria nos.

3, 4 and 5 are met. It is discussed that the diagnosis of secondary Sjögren’s syndrome can be considered confirmed once typical symptoms (such as dryness problems) and SS-A/Ro or SS-B/La antibodies are present. Under these circumstances, it is not mandatory to perform further examinations such as a lip biopsy.

  • Eye complaints At least one positive answer to one of the questions 1-3 in the questionnaire attached below.
  • At least one positive answer to one of the questions 4-6 in the questionnaire below.
  • Eye findings Positive Schirmer or Rose Begal test.
  • Tissue findings At least 1 lymphoid cell focus (>50 mononuclear cells) per 4 mm2 of salivary gland tissue
  • Salivary gland involvement At least one positive result in the following 3 tests: salivary gland scintigraphy, parotid sialography unstimulated salivary flow (< 1.5 ml15 minutes).
  • Scintigraphy of the salivary glands,
  • Parotian Dialogue Graphic
  • Unstimulated saliva flow (< 1.5 ml15 minutes).
  • Autoantibody detection At least one positive result: SS-A/Ro- or SS-B/La antibodies Antinuclear antibodies (ANA) Rheumatoid factors
  • SS-A/Ro or SS-B/La antibodies
  • Antinuclear antibodies (ANA)
  • Rheumatoid factors
  • Scintigraphy of the salivary glands,
  • Parotian Dialogue Graphic
  • Unstimulated saliva flow (< 1.5 ml15 minutes).
  • SS-A/Ro or SS-B/La antibodies
  • Antinuclear antibodies (ANA)
  • Rheumatoid factors

The main complaints of affected patients are dryness of the eyes, mouth and other mucous membranes. If these complaints occur and cannot be explained by allergies or similar, Sjögren’s syndrome should also be considered.

  • Eye involvement: Physiologically, the surface of our eyes is wetted by the tear film.

    The tear film consists of different components, which are released from different glands. If this tear film is insufficient, the image of the “dry eye” is created. The following characteristics are typical: If these complaints occur and cannot be explained by allergies or similar, then the Sjögren syndrome should also be considered.Dryness feeling of the eyes Foreign body feeling of the eyes (“sand in the eye”) Red and inflamed eyes increased tearing of the eyes

  • Dryness feeling of the eyes
  • Foreign body sensation of the eyes (“sand in the eye”)
  • Red and inflamed eyes
  • Increased tearing of the eyes
  • Mouth participation: Not only the lacrimal gland is affected, but also the salivary glands which are responsible for the secretion of saliva into the oral cavity (parotid gland, mandibular and lingual parotid glands).

    Here the following symptoms occur: Dryness in the mouth and throat Frequent drinking Inflammation of mucous membrane and gums

  • Dryness in the mouth and throat
  • Drink frequently
  • Inflammation of mucous membrane and gums
  • Other board memberships: Other mucous membranes of the body may also be affected: nose, trachea and bronchi. Organ involvement includes, for example: joints, muscles and lungs. Other organs are rarely or very rarely affected.
  • Dryness feeling of the eyes
  • Foreign body sensation of the eyes (“sand in the eye”)
  • Red and inflamed eyes
  • Increased tearing of the eyes
  • Dryness in the mouth and throat
  • Drink frequently
  • Inflammation of mucous membrane and gums

The suspicion is voiced by the doctor, usually an ophthalmologist and an ENT or dentist, on the basis of the symptoms described.

Antibody tests are carried out in order to be able to make a reliable diagnosis. These tests detect the autoantibodies SS-A and SS-B, which are typical for Sjögren’s syndrome. A detailed article about many different autoantibodies and the autoimmune diseases caused by them can be found at Autoantibodies

  • Have you been suffering for more than three months from daily, stressful dry eyes and mouth?
  • Do you often feel a foreign body (sand) in your eyes?
  • Do you use tear substitutes more than 3 times a day?
  • Have you been suffering from daily dry mouth for more than three months?
  • As an adult, did you suffer from recurrent or persistent swelling of oral salivary glands?
  • Are you forced to drink something to swallow dry food?

In addition to the classic symptoms of Sjögren’s syndrome, such as dry mouth and dry eyes, sufferers can suffer from a variety of other ailments.

Many patients suffer from headaches, which can be of varying severity. Migraine-like attacks with nausea and sensitivity to light can also occur in Sjögren’s syndrome. Hair loss may be a sign of iron deficiency in patients with Sjögren’s syndrome.

Dehydration of the skin and mucous membranes often leads to small bleedings, and blood clotting can also be disturbed. If a person regularly loses blood, this can manifest itself as an iron deficiency. However, hair loss can also be triggered by various drugs used in the treatment of Sjögren’s syndrome (for example, immunosuppressive or cytostatic drugs).

A causal therapy of Sjögren’s syndrome is still not possible today – the cause cannot be fought. Only the symptoms are treated. The various disciplines of medicine go hand in hand.

The ophthalmologist prescribes tear substitutes, so-called “artificial tears”, to keep the eyes moisturized. The dentist recommends saliva-boosting agents such as unsweetened lemon candy or mouthwashes. For joint complaints, painkillers or anti-inflammatory drugs can be administered.

In general, sufficient fluid intake of at least 1.5 liters daily should be ensured. Although there are a number of drug and non-drug approaches to symptomatic improvement, Sjögren’s syndrome is not yet considered curable. However, new therapeutic options have recently been explored to control disease activity and improve patients’ quality of life.

The new treatments are so-called biological therapies and drugs (“biologicals”), which act immunomodulatory. This means that drugs are used to attempt to modulate the immune system so that it is no longer active against the body’s own structures. Currently, no drug from this field has been approved for the treatment of Sjögren’s syndrome, but intensive research is currently underway in this area, so there is hope for improved treatment options in the near future.From a scientific point of view, homeopathic preparations do not offer an adequate substitute treatment for Sjögren’s syndrome and are by no means sufficient to prevent or slow down the progression of the disease.

However, alternative healing approaches can improve the quality of life and help to alleviate the symptoms. A homeopathic therapy can be planned and accompanied by an experienced homeopath. In case of inflammation and an acute worsening of the symptoms, a doctor must be consulted immediately.

To make a diagnosis, the doctor takes a blood sample, which is then tested for various laboratory values and antibodies. Typically, Sjögren’s syndrome is characterized by polyclonal hypergammaglobulinemia. This means that the immune cells in the blood produce increased levels of certain antibodies (gamma globulins).

This alone, however, does not prove the presence of Sjögren’s syndrome, as hypergammaglobulinemia occurs in many diseases, e.g. infectious diseases or chronic inflammatory bowel diseases. Due to the many antibodies in the blood, the blood sedimentation rate (BSG) is also increased. Patients may also have a deficiency of red blood cells (anemia), white blood cells (leukopenia) and/or blood platelets (thrombocytopenia).

50-80% of patients with Sjögren’s syndrome show positive detection of certain autoantibodies, the ANA (antinuclear antibodies). These are antibodies produced by immune cells and directed against the body’s own cell nuclei. Normally, antibodies protect the body from foreign substances such as bacteria or viruses.

A positive detection of ANA can therefore be an indication of an autoimmune disease in which the immune system mistakenly recognizes the body’s own structures as foreign and destroys them. Other autoantibodies that indicate Sjögren’s syndrome are SS-A antibodies and SS-B antibodies. These proteins target other components of the cell nucleus.

Antibodies against components of the salivary glands or so-called rheumatoid factors are also noticeable in some patients. The level of antibodies in the blood does not allow any conclusion to be drawn about the severity of the disease, as this depends primarily on the clinical symptoms. A positive laboratory result must always be evaluated together with the clinical symptoms in order to make the final diagnosis of Sjögren’s syndrome.

In almost 50% of those affected, the disease occurs on its own, i.e. without other concomitant diseases: so-called primary Sjögren’s syndrome. A secondary Sjögren’s syndrome is usually a rheumatic underlying disease (for example chronic polyarthritis). The exact cause is unknown.

A malfunction of the immune system is suspected. This seems to be directed against the body’s own cells, a so-called autoimmune disease. The Sjögren syndrome cannot be prevented.

It is a malfunction of the immune system, which turns against the own body and tries to fight it. Until today, the development of this autoimmune disease is not yet fully understood, so that no preventive measures can be taken. Sjögren’s syndrome is a chronic disease that cannot yet be cured.

This means that patients have to live with the disease throughout their lives. Nevertheless, Sjögren’s syndrome has a relatively good prognosis because the disease progresses very slowly. At the beginning of a primary Sjögren’s syndrome, the salivary and lacrimal glands are usually affected.

The tissue is attacked by white blood cells (lymphocytes) and eventually destroyed. This becomes noticeable by complaints in the eyes and mouth (dehydration). As the disease progresses, other organs, such as the skin, nervous system, joints and internal organs are also affected.

As a result, those affected often have a significantly reduced quality of life. The course of the secondary Sjögren’s syndrome, which occurs in the context of other diseases (e.g. rheumatoid arthritis or chronic hepatitis B), is determined by the underlying disease. Medication and adequate therapy (e.g. administration of artificial tear or saliva) can alleviate the symptoms and significantly improve the quality of life.

The disease is not fatal per se. Patients with Sjögren’s syndrome often die of cardiovascular diseases, infections or lymphomas (lymph gland cancer).In general, the prognosis for Sjögren’s syndrome is good, but depends on the accompanying diseases, such as joint involvement, etc. In most cases, Sjögren’s syndrome is benign, as the disease progresses very slowly despite the lack of cure to date, and therefore has a relatively favorable prognosis.

Accordingly, most patients have no reduced life expectancy. However, life expectancy depends largely on whether other organs are involved. In addition to the tear and salivary glands and various other glands (such as the sweat glands), the nervous system or internal organs can also be affected by the disease.

In particular, people who have Sjögren’s syndrome with lung involvement have a fourfold increased probability of dying prematurely from the disease. In addition, Sjögren’s syndrome significantly increases the risk of developing various malignant tumors of the lymph nodes (for example, non-Hodgkin’s lymphoma, MALT lymphoma, or marginal zone lymphoma). Sjögren’s syndrome can either progress slowly or in relapses.

Relapsing means that the disease activity is permanent, but the symptoms are not always equally pronounced. New inflammation triggers a “relapse” characterized by acute symptoms such as dryness around the eyes and in the mouth. Those affected then feel exhausted, powerless and tired.

Often, the relapse is preceded by greater physical exertion or excessive demands, but this does not necessarily have to be the case. The consumption of alcohol, caffeinated drinks (coffee and tea) or foods with a high sugar content can also cause the inflammatory activity in the body to flare up and trigger a relapse. In Sjögren’s syndrome, there are inflammatory processes in the body that are directed against glands and a number of other organs.

A healthy and balanced diet can improve the symptoms, support treatment and improve the patient’s quality of life. The main characteristic of the disease is dryness of the eyes and mouth. For this reason, patients should drink as much liquid as possible, avoiding caffeinated coffee, green and black tea, as these draw water from the body.

The same applies to alcohol and spicy foods. Sugary drinks also aggravate the complaints, which is why those affected should have recourse to water, unsweetened teas or highly diluted fruit juices. Sugar-free chewing gum or lollipops can stimulate the salivary production of the glands and are therefore a good choice.

Many people have to change their eating habits after being diagnosed with Sjögren’s syndrome. They often have one or more food intolerances (e.g. to gluten). Meals should consist mainly of The excessive consumption of meat, dairy products, processed foods and sugary foods should be restricted as much as possible.

This ensures that sufficient vitamins, minerals and trace elements can be absorbed by the body.

  • Lots of fresh vegetables,
  • Fish,
  • Olive oil and
  • Fruit exist.

Sjörgren’s syndrome is characterized by changes in the mucous membranes and skin. The disease gradually destroys the glands of the body.

As a result, the mucous membranes dry out and the skin becomes dry and flaky. Sjögren’s syndrome is often accompanied by purple-colored skin changes, inflammatory redness of the skin (annular erythema), wheals and itching. Some patients develop Raynaud’s syndrome as an accompanying symptom.

This leads to reduced blood circulation in the fingers, which causes the skin in the affected areas to turn white. Afterwards, the color changes to blue due to the lack of oxygen in the tissue and this reactively leads to increased blood circulation, the fingers turn red and hurt. Due to the typical tricoloring of the skin (white-blue-red), Raynaud’s syndrome is also known as the “tricolor phenomenon”.

This circulatory disorder can last for several hours and is often triggered by cold or stress in Sjögren’s patients. Patients suffering from Sjögren’s syndrome have the option of applying for a severely disabled card. The degree of disability (GdB) depends on the physical limitations in daily life.

Depending on the symptoms the person concerned suffers from, there are different scores that correspond to the GdB (e.g.30 points correspond to a GdB of 30). A medical expert determines the degree of disability individually. The exact cause of the Sjögren’s syndrome is still unclear, but there is growing evidence that, in addition to environmental influences and hormonal factors, genetic components also contribute to the development of the disease.

Nevertheless, the risk of passing on Sjögren’s syndrome to the child is relatively low at 1-3%. Since middle-aged women in particular are affected by the disease, it often happens that patients are or want to become pregnant. In the presence of Sjögren’s syndrome, the desire for a child should be carefully planned with the treating physician and the gynecologist, as the treatment of the disease usually has to be changed during pregnancy.

Many medications used in Sjögren’s syndrome must be discontinued because they are considered to be harmful to germs or the safety of these preparations during pregnancy is not sufficiently assured. Women with primary Sjögren’s syndrome have no increased risk of miscarriage or premature birth compared to the general population. In secondary Sjögren’s syndrome, this factor depends largely on the underlying disease.

In extremely rare cases, autoantibodies of the mother may be transferred to the fetal bloodstream during pregnancy. This can result in cardiac arrhythmia and the development of B-cell lymphomas (a malignant tumor of the lymph nodes) in the child. The presence of Sjögren’s syndrome does not mean that the affected women should not have children.

The risk of premature birth or miscarriage during pregnancy is not increased by the disease and the risk of passing the disease on to the child is relatively small. However, women who wish to have children should be aware that bringing up a child can be an extraordinary psychological and physical burden, especially if you are not physically fit. In any case, patients should discuss family planning with their treating physician so that medication can be discontinued before conception and an alternative treatment option can be considered during pregnancy.

Often an autoimmune disease like Sjögren’s syndrome occurs together with other diseases and vice versa. For example, more than 20% of patients with Hashimoto’s thyroiditis also suffer from Sjögren’s syndrome. If Sjögren’s syndrome occurs together with Hashimoto’s disease, it is called “secondary Sjögren’s syndrome” (as opposed to primary Sjögren’s syndrome, which occurs without concomitant diseases).

Hashimoto’s thyroiditis is also an autoimmune disease that causes chronic inflammation of the thyroid gland. As a result, the thyroid gland becomes underactive and patients feel exhausted and tired. The exact relationship between the two diseases is not yet fully understood, but in both cases autoimmune processes play an important role.